Durgadatta Pattanyak, Raghvendra Singh Patel, Animesh Kumar and Rajniti Prasad*

Department of Paediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi

*Corresponding Author: Rajniti Prasad, Department of Paediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi.

Received: August 14, 2023; Published: October 16, 2023

Abstract

Hemiconvulsion hemiplegia epilepsy (HHE) syndrome is a rare complication of prolonged focal seizures in children and usually idiopathic but may be associated with structural, infective, traumatic and degenerative diseases. We present two cases with initial presentation as left sided focal status epilepticus, later developing left sided hemiparesis with iron deficiency anemia. This is the first association of iron deficiency with hemiconvulsion-hemiplegia-syndrome illustrating the importance of testing for iron deficiency and neuroimaging in each case of status epilepticus. More importantly, these cases also raise the possibility that deficiency in iron could be a contributing factor in cases of HHE.

Keywords: Hemiconvulsion Hemiplegia Epilepsy (HHE); Febrile Seizure; Status Epilepticus; Iron Deficiency Anemia

References

1. Gastaut H., et al. “H.H.E. syndrome: hemiconvulsions, hemiplegia, epilepsy”. Epilepsia 1 (1959): 418-447.
2. Auvin S., et al. “Neuropathological and MRI findings in an acute presentation of hemiconvulsion-hemiplegia: a report with pathophysiological implications”. Seizure 16 (2007): 371-376.
3. Yamazaki S., et al. “Hemiconvulsion-hemiplegia-epilepsy syndrome associated with CACNA1A S218Lmutation”. Pediatric Neurology 45 (2011): 193-196.
4. Mondal RK., et al. “Hemiconvulsion, hemiplegia, epilepsy syndrome and inherited protein S deficiency”. Indian Journal of Pediatrics 2 (2006): 157–159.
5. Kenneth A Myers., et al. “Hemiconvulsion hemiplegia epilepsy in a girl with cobalamin C deficiency”. Epileptic Disorders 6 (2018): 545-550.
6. Lee C., et al. “Hemiconvulsion-hemiplegia-epilepsy syndrome as a presenting feature of L-2-hydroxyglutaric aciduria”. Journal of Child Neurology 21 (2006): 538–540.
7. Jonathan L Maguire., et al. “Association between iron deficiency anemia and stroke in young children”. Pediatrics 120 (2007): 1053-1057.
8. Byung Ok Kwak., et al. “Relationship between iron deficiency anemia and febrile seizures in children: a systematic review and meta analysis”. Seizure 52 (2017): 27-34.
9. Kumar RV., et al. “Correlation of serum level and selective blood indices in children with febrile seizures in Chittoor district, India”. International Journal of Contemporary Pediatrics 6 (2020): 1337-1340.
10. Kumari Pl., et al. “Iron deficiency as a risk factor for simple febrile seizures- a case control study”. Indian Pediatrics 1 (2012): 17-19.
11. , et al. “Iron Deficiency and Iron Deficiency Anemia in Children with First Attack of Seizure and On Healthy Control Group; A Comparative Study”. Iranian Journal of Child Neurology 8.3 (2014): 18-23.
12. Zareifar S., et al. “Association between iron status and febrile seizures in children”. Seizure8 (2012): 603-605.

Citation

Citation: Rajniti Prasad., et al. “Hemiconvulsion Hemiplegia Syndrome with Iron Deficiency Anemia in Children: A Report of Two Cases with Review of Literature". Acta Scientific Neurology 6.11 (2023): 22-25.

Copyright

Copyright: © 2023 Rajniti Prasad., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.